Massive Gastrointestinal Hemorrhage: A Deadly Presentation of Hemophagocytic Lymphohistiocytosis

Document Type

Conference Proceeding

Publication Date

5-1-2025

Publication Title

Am J Respir Crit Care Med

Keywords

anakinra, dexamethasone, etoposide, ferritin, lactate dehydrogenase, steroid, adult, adverse drug reaction, artificial embolization, blood transfusion, bone marrow biopsy, bone marrow transplantation, case report, clinical article, complication, computed tomographic angiography, computer assisted tomography, conference abstract, diagnosis, drug dose, drug therapy, duodenum ulcer, endoscopy, gastroduodenal artery, gastrointestinal hemorrhage, hematemesis, hematochezia, hemodynamics, hemophagocytic syndrome, hemostasis, human, human tissue, hyperinflammation, hypertriglyceridemia, hypofibrinogenemia, hypothyroidism, immunosuppressive treatment, male, melena, meningioma, multiple organ failure, nausea and vomiting, pancytopenia, primary hemophagocytic lymphohistiocytosis, push enteroscopy, secondary hemophagocytic lymphohistiocytosis, sepsis, splenomegaly, therapy, treatment protocol

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition characterized by dysregulated hyperinflammation resulting in multiorgan failure. While primary HLH can present in childhood, secondary HLH presents in adulthood instigated by acute illness (most commonly sepsis), malignancy, or autoimmune disorders. The presented case highlights the challenges of managing gastrointestinal hemorrhage in a young man with relapsing HLH. Case Presentation: A 27-year-old male with history of recently diagnosed hemophagocytic lymphohistiocytosis, hypothyroidism, and resected meningioma presented to the hospital due to intractable nausea, vomiting, and dark stools. He was diagnosed with HLH three months prior via bone marrow biopsy and started on dexamethasone and etoposide, the last dose administered two weeks prior to admission. Primary HLH workup was unremarkable for congenital etiology. Admitting labs and exam suggested HLH recurrence (Table 1): pancytopenia, ferritin levels greater than 47,000, LDH above 400, hypertriglyceridemia, hypofibrinogenemia, and associated febrility and splenomegaly on exam. He was started on anakinra, ruxolitinub, and dexamethasone. The hospital course was complicated by multiple episodes of acute gastrointestinal hemorrhage presenting as hematemesis and hematochezia. Two rounds of massive transfusion protocol were completed. Push enteroscopy revealed duodenal ulcers, the largest 12mm in diameter. Computed tomography (CT) angiography confirmed active hemorrhage, and the patient underwent embolization of the left gastric, pancreaticoduodenal, and the gastroduodenal artery at the junction of the second and third portion of the duodenum. Discussion: HLH is characterized by indiscriminate inflammation resulting in multiorgan failure. In most cases, hemodynamic collapse results in death. Gastrointestinal hemorrhage is a challenging complication of HLH with incidence and mortality estimated to be 12.2% and 66%, respectively. Endoscopy typically has minimal benefits due to diffuse transmural inflammation along the length of the gastrointestinal tract which can be accompanied by panenteric ulceration that is difficult to stabilize due to high-risk of recurrent bleeding. Initial management includes hemodynamic stabilization with blood transfusion and hemostasis. In this case the bleeding source was found via CT angiography with percutaneous embolization utilized to attain hemostasis. Following hemodynamic stabilization, the cornerstone of HLH management is immunosuppression through the use of high dose steroids and cytotoxic chemotherapy. Ultimately, patients undergo bone marrow transplantation once the inflammation is subdued. HLH with gastrointestinal bleeding presents a challenging case due to dysregulated inflammation leading to mucosal ulceration and large volume bleeds. Additionally, the treatment protocol potentially increases the risk of gastrointestinal bleeding or perforation by 40%, while also increasing the risk of propagating underlying infectious processes.

Volume

211

Issue

Supplement 1

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